Sickle Cell and Anemia
Clinical Reasoning
Pediatric Cancer and Intro to Hem/Onc
Histology
OMM and Implicit Bias
100

Pt has a positive Direct Coombs test. Describe what this means in terms of AIHA.

BONUS! They determine the binding is to the complement only. Identify the specific AIHA.

What is that this pt's blood agglutinates in the tube due to the presence of autoantibodies against the RBC's antigens? AIHA is when there is an immune response towards the RBCs which causes hemolysis of RBCs. There are two main types, warm and cold. There are multiple differences between the two, but the main concept is that warm agglutinin disease is when IgG Abs bind to RBC surface antigens in temperatures above 30 degrees Celsius, and cold agglutinin disease is when IgM Abs binds to RBC surface antigens in temperatures below 30 degrees Celsius. 

BONUS! If the DAT is only positive for the complement, they most likely have cold agglutinin disease (CAD) since the IgM Abs leaves Cd3 on the RBC surface for macrophage phagocytosis.

100

Identify the malignancy that is associated with smudge cells and the relation between smudge cells and prognosis.

What is CLL with smudge cells being negatively correlated to prognosis? The cells are lymphocytes that "smudge" during blood smear preparation due to improper proliferation. If there is a high percentage of smudge cells, the prognosis for survival is low.

iLAB

100

Identify the 4 classes of normal regulatory genes.

What are protooncogenes, tumor suppressor genes, regulators of apoptosis, and DNA repair? Mutations in any of these can be involved in the development of neoplasms. Some of these mutations can be due to random mutations, inherited, or environmental factors.

Obj. 12333

100

Identify the location of production of:

alpha and beta globulins

gamma globulins

plasma lipoproteins

What are the liver, plasma cells, and intestine/liver?

Obj. 6430

100

Describe the difference between explicit and implicit bias as well as where implicit bias comes from.

BONUS! Name the individual who defined microaggressions. 

What is explicit bias is a belief of a certain race or individual group that is known and conscious whereas implicit bias is one that is unconscious and individual, but comes from shared social experiences. 

BONUS! Chester Pierce

iLAB

200

Pt presents to the office to hear their results from their CBC with differential. Identify if the pt should be concerned about Leukemia or Infection based on these results.

RBC: 4.0 x10^6/mm^3

WBC: 50 x 10^3/mm^3

Segmented Neutrophils: 30%

Band Neutrophils: 10%

Eosinophils: 1%

Lymphocytes: 20%

Basophils: 0.5%

What is this pt be more concerned about infection due to the increase of band Neutrophils? A left shift can be seen in severe infections which cause an increase in WBCs. One of the main differences between left shift and Leukemia is the absence of Basophilia.

Obj. 6408

200

Identify the condition that is most commonly associated with Heinz bodies and the condition most commonly associated with Howell Jolly bodies.

What are G6PD deficiency and lack of functional spleen?

iLAB

200
Explain Differentiation and how it plays into grading and/or staging.


BONUS! Identify the neoplasm masses that are composed of disorganized, benign cells that are indigenous to the involved site.

What is that differentiation is based on how alike or unalike the parenchymal cells of a neoplasm are to the normal cells morphologically/functionally and plays a huge part in grading? Differentiation can range from well-differentiated to poorly differentiated where the least differentiated cells represent those the furthest morphologically/functionally from normal cells. This also means that the least differentiated, the higher grade, more malignant. 

BONUS! Hamartoma

Objs. 12329 and 12330

200

Pt's blood smear indicates anisopoikitocytosis and further studies confirm severe hemolytic anemia. If the cells appear small with thorny projections, they would be classified as...

What are Acanthocytes? Anisopoikitocytosis is when the RBCs vary in size (aniso) and shape (poikito) which can be seen in any severe anemia. RBCs that are small with thorny projections are acanthocytes which are seen in hemolytic anemia and cirrhosis. If the cells had more of spinous processes like a burr of a cowboy boot (if those still exist), they would be considered burr cells(echinocytes).

iLAB

200

6 y/o pt presents to the clinic with an antalgic gait. They seem to be shortening their time in the stance phase and started during rough soccer practice that afternoon. Identify whether the physician should order a CBC with diff and ESR. Vitals are: 

BP: 120/80

HR: 90

RR: 14

Temp:  98.5


What is that the physician should not need to order ESR due to suspected overuse causing the antalgic gait? ESR should be obtained when infection, inflammatory arthritis, or malignancy is suspected, but since this individual had rough soccer practice that day it is most likely from overuse.

Obj. 6447

300

In testing a pt for sickle cell, hemoglobin electrophoresis was completed. This test showed an increase in Hb with 2 alpha subunits and 2 deltas. Additionally, there is a decrease in HbA. Identify what these results mean in terms of Sickle cell. 

BONUS! Describe the pathophysiology in the production of sickle cells.

What is that this pt has sickle cell disease trait? These results indicate a decrease in beta globin production so the HbA concentration is reduced. Since there is a decrease in beta-globin and HbA production, there is a compensatory increase in HbA2 production.

BONUS! In hypoxia, dehydration, and acidosis, there is a preference for taut Hbs which promotes the polymerization of HbS. There is a change in the 6th amino acid from Glutamic acid to Valine causing an increase in hydrophobic interactions.

Obj. 6402 

300

 Newborn pt presents with umbilical bleeding 1 day after a home birth. Studies show:

Normal PLT

Prolonged PT

Prolonged PTT

Identify the main choices of bleeding disorders in the pt.

BONUS! Use the former name of this condition (AKA old name based on the cohort it is found in)

What is Vitamin K Deficiency? Since the platelet count was normal, any type of thrombocytopenia is ruled out. Next, since both PT and PTT were prolonged, this meant that it had to be a condition that affected both intrinsic (PTT) and (PT) pathways. In newborns that were born at home and you are not certain if they received proper after-birth care,  it is safe to lean towards Vit K deficiency. 

BONUS! The Hemorrhagic disease of the newborn

iLAB

300

2 y/o pt presents to the office with a watery eye and bruising. The mother states that the watery eye has been present for months, and the pt was vomiting with a low-grade fever a week before the visit. Upon examination, it was found that the pt had splenomegaly and was sent to get lab work. The CBC with diff showed:

WBC: 21.7 x 10^9/L

Band cells: 25%

Platelets: 69 x 10^9/L

RBC: 3.3 x 10^12/L

Identify the top two differentials for this individual in regards to malignant conditions based on the symptoms and CBC with diff.

What are ALL and AML? This pt had symptoms of Leukemia with increased WBCs/blast %, splenomegaly, and bruising. In this case, the individual has AML and the splenomegaly is due to the increased destruction of RBCs and infiltration of leukemic cells. 

FUN FACT! This was based on my case! I had shown zero symptoms besides those above. My WBC count was so high, that my eye was watering to release all of the extra cells.

300

Identify this cell. its function, and location in the body. (look at Jeopardy Onenote link for image)

What is a megakaryocyte which produces platelets and is located only in the bone marrow due to its ability to clog that capillaries from its size?

Obj. 6433

300

Describe the characteristics of a good squat.

What are flat feet, shoulders behind the knees, and mostly upright trunk?


iLAB

400

18 y/o female pt with anemia has their results shown below. Define their anemia and the RBC shape that will most likely be seen.

RBC = 4.4 x 10^6/mm^3

Hct = 30%

Hb = 10.6 g/dL

Ferritin =10 ug/L

Iron = 48 ug/dL

TIBC = 350 ug/dL

% saturation = 28%


What is iron deficiency anemia with target cells? MCV = 68% which classifies a microcytic anemia. From there, the iron is decreased, ferritin is decreased, % saturation is decreased, and TIBC is increased. 

iLAB

400

6 y/o pt presents to the office for a checkup regarding their cold. Pt's mother states that the symptoms have gone away since the last visit, but has noticed "purple-red" dots on the pt's legs as well as a bad case of epistaxis yesterday. Bloodwork was completed on the pt and found everything to be normal except for the platelet count which was 15 x 10^3/mm^3.  Identify the most likely diagnosis for this pt and the tx.

BONUS! Describe the pathophysiology behind this disorder.

What is ITP with treatment involving steroids? ITP is most commonly seen after a viral infection in children and usually resolves on its own within 2 months as long as they are not severe. In this case, the pt has active bleeding as well as a severely low platelet count, so they need steroids rather than observation like normal in acute ITP. Symptoms lean towards ITP as well as the lab results, especially since there were no symptoms of the TTP Triad (Anemia, Neurological impairment, thrombocytopenia).

BONUS! In ITP there are autoantibodies that form against GPIIb/IIIa receptors on platelets which leads to destruction and thrombocytopenia.

400

Pt is found to have a colonic adenocarcinoma. Upon investigation of the biopsy, the cells moderately represent normal cells and pass into the submucosa. Additionally, the mass is found to have not spread to other tissues, but some cells have been found in 2 lymph nodes. Based on the TNM scale and grading describe this neoplasm

BONUS! Identify the change in the description if these cells had also been found in the stomach.

What is that this pt most likely has a Grade 2 T3 N2 M0 neoplasm? The cells in the biopsy showed moderate differentiation meaning that the grade is about a 2. The cells have penetrated the outer layers of the wall (T3), spread into 2 lymph nodes (N2), and have not spread into other tissues (M0).

BONUS! The only change would be from M0 to M1

Objs. 12330 and 12332

400

Identify the important steps in leukopoiesis where:

Primary granules are produced

Last mitotic stage 

BONUS! Identify the last stage in erythropoiesis and monopoiesis that mitosis is possible



What are the promyelocyte stage and myelocyte stage?

Objs. 6431 and 6432

BONUS! Polychromatiphilic erythroblast (erythropoiesis) and Promonocyte (monopoiesis)


400

8 y/o pt presents to the office for a normal check-up. While walking to the exam room, the MA notices that the pt seems to have a downward tilt toward their right leg when in their stance phase and informs the physician. Having been a studious medical student while in school, they ask the pt to stand on their left leg to observe the pt's stance phase. Describe the test the physician performed and explain the results of this pt.

What is that this physician suspected a Trendelenburg gait in the pt and was testing for gluteus medius weakness in the left leg? Normally, when walking, the ipsilateral hip should drop while in the stance phase due to the contraction of the gluteus medius. This is counteracted by a contralateral elevation of the hip resulting in a balanced pelvis in the stance phase. 

Obj2. 6449 and 6452

500

Central sensitization is defined as increased responsiveness of nociceptive neurons in the CNS to their normal or subthreshold afferent input. During this process, there are many chemicals involved. Describe the beginning step that needs to occur during stimulation and the transcriptional changes that occur. 

BONUS! Describe the roles that Glutamate, BDNF, and NMDA play in this process.

What is that there is a "wind-up" period and transcriptional changes include an upregulation in depolarization ion channels and down-regulation in hyperpolarization ion channels?


BONUS! These substances have varying roles in central sensitization. Glutamate is an excitatory NT which activates AMPA receptors which result in a decrease in Mg2+inhibition on NMDA receptors. The BDNF molecules will phosphorylate NMDA/AMPA which allows for NMDA to increase Ca2+ for depolarization, and NT release.

Obj. 6400

500

Pt presents to the ED via ambulance due to their partner calling 911. The partner states that the pt has been sick with a fever and started hallucinating. Pt's urine is found to be dark and CBC showed a low platelet count. Reticulocyte count is high, and a blood smear was taken which showed spherocytes and fragments of RBCs. Identify the most likely diagnosis of this pt if their coagulation studies were found to be normal.

BONUS! Identify the tx in this individual.

What is TTP? since the platelet count was low, it is necessary to look at platelet disorders such as ITP and TTP. The symptoms shown here represent the pentad of TTP: RAFTN(Renal insufficiency, Anema (MAHA), Fever, Thrombocytopenia, Neurological impairment).

BONUS! Tx includes glucocorticoid steroids, Monoconal Ab, and/or plasma exchange


Objs. 6478 and 6480

500

Pt presents to the office with c/o fatigue, chills, and night sweats. Upon examination, it was found that the pt has a nontender cervical lymphadenopathy. A blood smear was taken and showed a diffuse sheet of B cells with high proliferation. Identify the most likely Lymphoma this pt has.

What is Diffuse Large B cell Lymphoma(DLBCL)? The B symptoms and non-tender cervical lymphadenopathy indicate Lymphoma. And then the blood smear showed B cells proliferating in a diffuse sheet, pointing to DLBCL.

Obj. 6465

500

Identify what this organ is and the difference between the white pulp and red pulp. (look at Jeopardy link for picture)

BONUS! Identify the parts of the white pulp and the contents of each.

What is the spleen where the white pulp is the location of the immune response and the red pulp is the location of blood filtration? 

BONUS! The layers of the white pulp include the germinal center (activated B cells), mantle zone(densely packed B cells and macrophages), and marginal zone(B cells, macrophages, dendritic cells) from inside to outside. Additionally, there is a central arteriole and PALS(T cells).

Obj. 6435

500

Describe the BLT technique on a plantarflexed right calcaneus.

What is the pt is supine with the physician on their right side holding the pt's forefoot and calcaneus whilst placing the pt's right knee and hip in flexion? After this position is completed, the physician needs to externally rotate and abduct the pt's right femur, slowly lean back to disengage the calcaneus, and plantarflex the pt. Overall, just remember easy DEB (disengage, exaggerate, balance all into ease)

iLAB